• Title/Summary/Keyword: Patients with hemophilia

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Experience of Hemophilic Pseudotumor Developed at the Fracture Site of the Proximal Phalanx of the Hand (수부 근위지골 골절 수술 후 발생한 혈우병성 가성종양의 치험례)

  • Park, Ji Ung;Kim, Chang Yeon;Shin, Jong In;Cho, Sang Hun
    • Archives of Plastic Surgery
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    • v.34 no.4
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    • pp.516-519
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    • 2007
  • Purpose: Hemophilia is one of the most common bleeding disorder, and circulating levels of factor VIII or IX are closely related to the severity and frequency of the bleeding. The severity of hemophilia is classified to be severe, moderate, and mild when the factor level is less than 1%, between 1% and 5%, and between 5% and 25%, respectively. Hemophilic pseudotumor is a rare complication occurring in 1 - 2% of patients with factor VIII or IX deficiency. If the treatment is delayed, it would cause disabling and life threatening results. Methods: We experienced hemophilic pseudotumor developed at the fracture site of the proximal phalanx of the hand in a hemophilic B patient. Hemophilic pseudotumor was successfully treated with perioperative factor replacement and surgical intervention included excision and autologous bone graft. Results: Hemophilic pseudotumor was healed with complete regression, and no specific complication was developed. Conclusion: When we accounter hematoma like lesion after surgery unpredictably, we must consider hemophilic pseudotumor and make a accurate diagnosis with preoperative hematologic screening and various imaging study. Subsequently, adequate perioperative supplement of concentrate and surgical intervention brings to the satisfactory result without recurrence.

Factor XI deficiency and orthognathic surgery: a case report on anesthesia management

  • Lee, Soo Eon;Choi, Yoon Ji;Chi, Seong-In;Kim, Hyun-Jeong;Seo, Kwang-Suk
    • Journal of Dental Anesthesia and Pain Medicine
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    • v.15 no.1
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    • pp.25-29
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    • 2015
  • Factor XI deficiency (Hemophilia C) is a very rare autosomal recessive bleeding disorder. Patients with factor XI deficiency do not typically show any spontaneous bleeding or specific symptoms. Sometimes those who have this disorder are identified during special situations such as trauma or surgery. Orthognathic surgery is particularly associated with a high bleeding risk. Therefore, great care must be taken when treating patients with bleeding disorders such as factor XI deficiency. There are a few reports that address the management of patients with bleeding disorders during orthognathic surgery. The current report describes a patient with factor XI deficiency who underwent Le Fort I osteotomy together with bilateral sagittal split osteotomy. The patient's condition was assessed using both rotation thromboelastometry ($ROTEM^{TM}$) and noninvasive measurements of total hemoglobin (SpHb) using Masimo Radical 7 (Masimo Co. CA, USA).

Radiosynovectomy (방사성 활막절제술)

  • Kim, Deog-Yoon
    • Nuclear Medicine and Molecular Imaging
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    • v.40 no.2
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    • pp.127-131
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    • 2006
  • Radiosynovectomy has been used as an effective treatment in patients with resistant synovitis after failure of long-term medication and intraarticular steroid injection. Although $^{90}Y$ silicate/citrate, $^{186}Re$ sulfide, and $^{169}Er$ citrate were approved in Europe for the appropriate radiopharmaceuticals for radiosynovectomy other radionuclides such as $^{32}P$-chromic phosphate, $^{165}Dy$-ferric hydroxide macroaggregate, $^{188}Rh$-microspheres, $^{153}Sm-particulate, and $^{166}Ho$-ferric hydroxide macroaggregate have been used in many countries. Reported success rates range from 40% to 90% for the different joints and underlying disease. In Korea, $^{188}Re$-tin-colloid and $^{166}Ho$-chitosan complex are now using as the major radionuclides in radiosynovectomy with good clinical results. A study on radiation synovectomy using $^{188}Re$-tin-colloid for patients with Korean rheumatoid arthritis shows the treatment resulted in the improvement of arthritis and well tolerated in our study, the radiosynovectomy with $^{166}Ho$-chitosan complex in 53 hemophilic patients markedly decreased intra-articular bleeding frequency and need for coagulation factor. This review inculdes general priciples in the application of radiosynovectomy and the clinical experience in Korea.

Hemophiliac Patient with Hemarthrosis and Physical Therapy (혈우병 환자의 관절혈증과 물리치료)

  • Lee, Woo-Hyung
    • The Journal of Korean Academy of Orthopedic Manual Physical Therapy
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    • v.16 no.1
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    • pp.43-49
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    • 2010
  • Purpose : The purpose of this study was to understanding of a hemophiliac with hemarthrosis and provided information of rehabilitative therapy. Methods : The General understanding and rehabilitative therapy of a hemophiliac with hemarthrosis considered the matter carefully using a related publications and a internet searching investigation. Results : From the physical therapeutic method for pain alleviation, decreasing edema, ability of muscular strength and control, range of motion(ROM) in joint, prevention of damage on hemorrhaged joint in the hemophiliac with hemarthrosis, rehabilitative therapy for exercise of ROM, muscular strength, proprioceptive sense was most effective for patients. Conclusion : Physical therapists known the rehabilitative therapy should helped a hemophiliac with hemarthrosis to prevent from permanent change.

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Femoral Neuropathy due to Iliacus Muscle Hematoma in a Patient on Warfarin Therapy

  • Kong, Woo-Keun;Cho, Keun-Tae;Lee, Ho-Jun;Choi, Jae-Sung
    • Journal of Korean Neurosurgical Society
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    • v.51 no.1
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    • pp.51-53
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    • 2012
  • Spontaneous hematomas of the iliacus muscle are rare lesions and these are seen in individuals receiving anticoagulation therapy or patients with blood dyscrasias such as hemophilia. It can cause femoral neuropathy and resultant pain and paralysis. Although there is no clear consensus for the treatment of femoral neuropathy from iliacus muscle hematomas, delays in the surgical evacuation of hematoma for decompression of the femoral nerve can lead to a prolonged or permanent disability. We report here on a rare case of a spontaneous iliacus muscle hematoma that caused femoral neuropathy in a patient who was taking warfarin for occlusive vascular disease and we discuss the treatment.

Clinical Significance of Coagulation Screening Tests and Platelet Counts in Children Undergoing Endoscopy (소아에서 내시경 검사의 출혈 위험 예측 인자로서 혈액 응고 검사 및 혈소판 검사의 의의)

  • Lee, Eun-Hye;Yang, Hye-Ran;Ko, Jae-Sung;Seo, Jeong-Kee
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.13 no.1
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    • pp.23-29
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    • 2010
  • Purpose: The aim of this study was to assess the benefits of routine pre-endoscopy coagulation screening tests and platelet counts in Korean children. Methods: Between March 2004 and December 2009, children who underwent gastrointestinal endoscopy for the evaluation of various gastrointestinal symptoms were included. All of the subjects included in the study also underwent routine coagulation screening and platelet count determinations prior to endoscopy and biopsy. The clinical records and laboratory tests were retrospectively reviewed in all patients. Results: One hundred sixty-two of 1,476 (11%) patients who underwent endoscopy had abnormal results on pre-screening coagulation tests. Fourteen patients underwent coagulation factor assays due to abnormal clotting results in consecutive tests or due to clinical evidence of a bleeding tendency. Seven patients were diagnosed with factor XII deficiency, one patient was diagnosed with von Willebrand disease, one patient had von Willebrand disease and factor XII deficiency, and one patient was presumed to have mild hemophilia. The remaining 4 patients had normal results with the factor assays. The results of platelet counts were normal with the exception of 1 patient. No patient had significant bleeding during the endoscopic procedures, despite abnormal pre-endoscopic coagulation tests. Conclusion: Routine coagulation screening tests and platelet counts revealed abnormal results in some patients. Most of the patients with abnormal clotting were shown to have a factor XII deficiency, which had no significant associated bleeding tendencies; the other patients were diagnosed with hemophilia or von Willebrand disease. Therefore, although abnormal pre-endoscopic coagulation is not always related to significant bleeding complications, pre-endoscopic coagulation screening may be useful in some children in predicting the risk of bleeding tendency during endoscopic procedures.

The Effect of Radiation Therapy on hemophilic Athropathy (혈우병성 관절증에 대한 방사선 치료의 역할)

  • Kang Jin Oh;Hong Seong Eon;Kim Sang Gi;Shin Dong Oh
    • Radiation Oncology Journal
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    • v.23 no.2
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    • pp.106-110
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    • 2005
  • Purpose : Repetitive bleeding into the joint space is the cause of debilitative hemophilic arthropathy. To interrupt this process, we treated the hemophilic patients suffering from repetitive joint bleeding with radiation therapy. Materials and Methods : From 1997 to 2001, a total of 41 joints from 37 hemophilic arthropathy patients were treated with radiation therapy at Kyung Hee University Hospital. The treated joints were 35 ankles, 3 knees and 3 elbows, respectively. The age of the patients ranged from 4 to 27 years (median age: 11 years). The radiation dose ranged from 900 cGy to 2360 cGy (median dose: 900cGy). The fraction size was 150 cGy, 180 cGy or 200 cGy. The number of bleeding in one year before and after radiotherapy was compared. Results : There was a tendency of frequent bleeding for the Patients younger than 11 (p=0.051) but there was also a tendency for more improvement in this group (p=0.057). The number of joint bleedings was related with joint pain (p=0.012) and joint swelling (p=0.033) but not with the Arbold-Hilgartner stage (p=0.739), cartilage destruction (p=0.718) and synovial hypertrophy (p=0.079). The number of bleeding was reduced in thirty-three cases, and eight cases showed no improvement after radiation therapy. The average number of bleeding in a month was 2.52 before radiotherapy, but this was reduced to 1.4 after radiotherapy (p=0.017). Conclusion : Radiation therapy was effective for the hemophilia patients with repetitive joint bleeding to decrease the bleeding frequency and to prevent hemophilic arthropathy.

Arthroscopic Synovectomy of the Knee in Hemophilic Patients (혈우병성 슬관절염의 관절경적 활액막 제거술)

  • Bae, Dae-Kyung;Yoon, Kyoung-Ho;Kim, Hee-Seon;Kim, Seung-Hwan;Chung, Sun-Teak
    • Journal of the Korean Arthroscopy Society
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    • v.6 no.2
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    • pp.177-182
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    • 2002
  • Purpose : To validate the arthroscopic synovectomy for the treatment of hemophilic knee. Materials and Methods: From January 1996 to January 2001, 28 arthroscopic synovectolny were performed in 26 patients with hemophilic arthropathy of the knee. The mean age was 17.8 years. The mean follow-up period was 3 years 11 months. We used six portals (two anterior, two suprapatellar and two posterior) and posterior trans-septal portal in all cases. Result : The mean frequency of hemarthrosis was 4 times per month preoperatively and 2 postoperatively. The mean amount of factor replacement was 4,633 units preoperatively and 1,505 postoperatively. The mean range of motion was $112^{\circ}$ preoperatively and $107^{\circ}$ postoperatively. On radiographic evaluation, three cases were progressed at the latest follow-up. On the subjective evaluation, significant or moderate improvement were in 19 cases $(68\%)$, and no improvement or deterioration in 9 cases $(32\%)$. Conclusion : With complete synovectomy through the appropriate arthroscopic portals, arthroscopic synovectomy of the knee in hemophilic patients is the successful method in decreasing bleeding episodes, amount of factor replacement, knee pain and preventing or delaying onset of end-stage hemophilic arthropathy.

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Fundamentals and Research Trend of Laser Acupuncture (레이저침의 원리와 연구동향 분석)

  • Kim, Hyun-Ho;Nam, Dong-Woo;Lee, Sang-Hoon
    • Journal of Acupuncture Research
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    • v.26 no.6
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    • pp.21-30
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    • 2009
  • Objectives : Being introduced in the 1980s, laser acupuncture studies have been steadily carried on such as its thermal effects on human body. However, the mechanisms of effect are not yet clearly explained. Generally, laser acupuncture could be favorable for pediatric patients or people under special conditions (hemophilia, needle-phobia, pathogen-carriers, etc.) because of lower risk of infection, bleeding, pain or fainting. But there are still problems for using laser acupuncture equivalently as the traditional metal needle. The goal of this study is to review the fundamentals, history and present trend of laser acupuncture study, and to suggest how to develop laser acupuncture as an oriental medical device. Method : We performed literature search using PubMed(http://www.ncbi.nlm.nih.gov/pubmed) and Scopus(http://www.scopus.com). Search conditions are laser acupuncture, laser needle, LLLT(as title or abstract) or Litscher G(as author). Main target studies are physical fundamentals, clinical trials, physiological results and device proposals. Results : Many research have shown the similarities of laser acupuncture and traditional metal acupuncture and have reported clinical and physiological effects of laser acupuncture. But, still laser acupuncture is not widely used in Korea due to high-cost of equipments and the lack of clear understanding of laser devices. Additionally, there are still challenges in standardization and device development. Conclusions : Frequencies, laser modes, waveguides, adaptor types, polarization and signal modulations are suggested as future study subjects on laser acupuncture. More various studies and efforts for device development have to be performed to provide oriental medical doctors with evidence based confidence about usage of laser acupuncture.

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